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Clinical Characteristics of Patients With HNF1-alpha MODY: A Literature Review and Retrospective Chart Review.


ABSTRACT: The clinical manifestation of hepatocyte nuclear factor-1-alpha (HNF1-alpha) maturity-onset diabetes of the young (MODY) is highly variable. This study aims to investigate the clinical characteristics of patients with HNF1-alpha MODY in general, by geographical regions (Asian or non-Asian), HNF1-alpha mutations, and islet autoantibody status. A literature review and a chart review of patients with HNF1-alpha MODY were performed. The means and proportions from studies were pooled using the inverse variance method for pooling, and subgroup analyses were performed. A total of 109 studies involving 1,325 patients [41.5%, 95% confidence interval (CI): 35.2, 48.1; male] were identified. The mean age of diagnosis was 20.3 years (95% CI: 18.3-22.2), and the mean glycate

SUBMITTER: Zhao Q 

PROVIDER: S-EPMC9252268 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature